Angioedema

Angioedema refers to swelling that occurs in the tissue just below the surface of the skin. It generally results from an allergic reaction to either a food or medication; it may be a sign, though, of an underlying condition such as leukemia or Hodgkin's disease. There are two basic types of angioedema:

• Hereditary angioedema (HAE), which is genetic and tends to recur
• Acquired angioedema (AAE)

The onset of angioedema varies widely, taking anywhere from minutes to hours to develop. Interestingly, angioedema may affect an area on one side of the body but not on the other.

Signs and Symptoms

Common symptoms of angioedema include:

• Burning, painful, swollen areas; usually not itchy; often affects eyelids, lips, tongue, throat, palms, soles, or genitalia
• Hoarseness, tight or swollen throat, breathing trouble
• Discolored patches or rash on the hands, feet, face, or genitals
• Vomiting, abdominal pain, diarrhea, and reduced appetite
• Swelling in or around the eyes
• In a form called angioedema-eosinophilia syndrome, hives, itching, fever, muscle pain, decreased urine, weight gain, and high white blood cell count occur

What Causes It?

Sometimes the cause is difficult to identify. An angioedema reaction (AAE in particular) may be caused by allergies to foods, dyes, or pollen, or in reaction to certain medications, including nonsteroidal anti-inflammatory drugs (NSAIDs, such as ibuprofen) and angiotensin-converting enzyme (ACE) inhibitors (such as captopril, lisinopril, enalapril). Conditions such as leukemia, Hodgkin's disease, and connective tissue disorders (such as systemic lupus erythematosus) may also trigger angioedema.

Who's Most At Risk?

These factors increase the risk for angioedema:

• Experiencing injury, sudden temperature change, stress, or anxiety
• Exercising intensely
• Undergoing a dental procedure
• Going through puberty or menstruating
• Having had a body-wide allergic reaction in the past
• Having ovarian cysts
• Taking ACE inhibitors (such as captopril, lisinopril, enalapril)

What to Expect at Your Provider's Office

Your healthcare provider will perform a physical exam and ask about your symptoms. Blood and urine tests may help pinpoint the cause of the angioedema.

Treatment Options

Prevention
You should eliminate any known or suspected triggers. Allergy testing with a trained specialist may help identify inciting agents. If you are prone to angioedema, you should wear a Medic Alert bracelet that notes this condition.

Treatment Plan
The first priority is to ensure that the airway is open and that breathing is not impaired. The next steps include identifying and removing the trigger as well as relieving other symptoms. Infrequent attacks can be managed as they arise. Frequent attacks may require ongoing treatment, perhaps with an allergist, dermatologist, or other specialist to try to avoid recurrences.

Drug Therapies
Several medicines may help prevent or relieve attacks. These include epinephrine, antihistamines, and corticosteroids. Children respond better to treatment with these medications than adults do. Acute attacks tend to clear up within four days with or without medication.

Complementary and Alternative Therapies
In a severe attack, standard emergency medical care should be administered immediately to open airways and stabilize the condition; no new substances, including herbs or supplements, should be introduced during an acute attack.

Long-term nutritional and herbal support used in between attacks may help reduce or prevent angioedema. In addition, herbs and supplements may help alleviate mild symptoms, particularly for chronic and recurring forms. Homeopathic remedies may help alleviate mild symptoms and reduce the frequency and severity of episodes.

Nutrition
Certain foods and food additives may trigger angioedema in people who are susceptible. You should eliminate any foods or food additives that trigger symptoms. The following are the most common food triggers:

• Seafood
• Nuts
• Legumes
• Eggs
• Chocolate
• Milk
• Berries

Certain individuals may have a reaction in response to:

• Citrus fruits
• Sulfites—used as an antioxidant or freshening agent (preservative) in many foods and beverages
• Yellow dye No. 5 (also called tartrazine)—those who react to aspirin or other NSAIDs are more susceptible to having an allergy to yellow dye No. 5

Healthcare providers can help identify food triggers by:

• Collecting detailed information about your diet
• Performing skin tests for sensitivity to certain substances
• Cautiously testing suspected triggers
• Monitoring symptoms as foods are eliminated from your diet then slowly re-introduced one at a time

If you have gastrointestinal symptoms (abdominal pain, vomiting, diarrhea, or reduced appetite), this may be an indication that you absorb antigens (food triggers) more easily than others through the stomach and intestines; in that case, you may benefit from a diet that eliminates common dietary antigens (as listed above) even if you do not have a specific, identifiable food allergy.

• Quercetin (a naturally occurring flavonoid) may reduce the likelihood of an allergic reaction. The recommended amount is generally 200 to 400 mg three times a day before meals. If you are sensitive to citrus or take calcium-channel blockers to treat high blood pressure, you should avoid citrus-based forms of flavonoids.
• Bromelain (Ananas comosus, an enzyme derived from pineapple)—used by some clinicians to help reduce inflammation; some recommend its use with turmeric (Curcuma longa), which may enhance the effects of bromelain.

Herbs

• Devil's claw root (Harpagophytum procumbens) — may be recommended by an herbal specialist to reduce inflammation and skin lesions
• Ginkgo biloba extract — used as an anti-allergenic agent and anti-inflammatory, although in very rare instances, ginkgo has been reported to cause an allergic reaction of the skin.
• Goldenseal (Hydrastis canadensis) — has been used for gastrointestinal symptoms such as stomach pain, diarrhea, and reduced appetite that may accompany a severe allergic reaction; some clinicians also recommend goldenseal for those with food allergies to prevent or lessen reactions
• Licorice root (Glycyrrhiza glabra) — has been used traditionally to diminish inflammation in the case of an allergic reaction; may also normalize immune function

Prognosis/Possible Complications

If angioedma affects the throat, the airway passage could be blocked, thereby creating a life-threatening situation. It is also possible that the angioedema may develop into anaphylaxis, which will require emergency medical care to maintain breathing, blood pressure, and heart function and to reverse the reaction.

Following Up

After an attack, it's important to identify and avoid any triggers and to treat any underlying condition.

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